How should I manage a patient with interstitial lung disease who presents with acute exacerbation?

Management of a patient with interstitial lung disease (ILD) presenting with an acute exacerbation requires prompt assessment and supportive care tailored to the severity of respiratory compromise. Initial management should focus on identifying and treating potential reversible causes such as infection or heart failure, while providing optimal oxygen therapy to correct hypoxia, avoiding excessive oxygen that may worsen hypercapnia if present. Oxygen saturation should be monitored closely using pulse oximetry, targeting appropriate saturation levels based on individual patient factors ¹.

Systemic corticosteroids are commonly used in acute exacerbations of ILD, although evidence is limited and their use should be guided by specialist input, particularly from respiratory physicians experienced in ILD management ¹. Mechanical ventilation, including non-invasive ventilation, is generally not recommended routinely due to poor outcomes in ILD patients with life-threatening respiratory failure; discussions about ventilation should occur early and involve the patient and family ¹.

Supportive care includes symptom relief, management of comorbidities, and consideration of palliative care involvement to address psychosocial needs and quality of life ¹. Referral to an ILD specialist nurse can provide additional support and information throughout the care pathway ¹.

Recent literature highlights that acute exacerbations of ILD, including those associated with rheumatic diseases, are characterized by rapid respiratory deterioration and often require intensive care support. The pathogenesis involves acute lung injury superimposed on chronic fibrosis, and treatment strategies emphasize immunosuppression (commonly corticosteroids) and supportive care, although evidence remains limited and outcomes are generally poor (Luppi et al., 2022; Charokopos et al., 2022). Rheumatoid arthritis-associated ILD exacerbations may require tailored immunomodulatory therapy (Akiyama and Kaneko, 2022).

In summary, management integrates guideline-based supportive care and oxygen therapy, cautious use of corticosteroids, avoidance of routine mechanical ventilation, and specialist multidisciplinary input, while recent studies underscore the severity and complexity of acute ILD exacerbations and the need for individualized immunosuppressive strategies in certain subtypes (e.g., rheumatic disease-associated ILD) ¹; (Luppi et al., 2022; Akiyama and Kaneko, 2022; Charokopos et al., 2022).